Abstract

Thrombotic microangiopathy (TMA) is a rare disorder that can occur secondary to steroid pulse therapy in hematopoietic stem cell transplant recipients. But the association between TMA and steroid pulse therapy among nephritis cases is not well known. We present a case of 75-year-old woman with rapidly-progressive glomerulonephritis. A week ago, she saw a local doctor for low appetite and fatigue. Blood biochemistry showed BUN 196 mg/dL and serum Cr 6.42 mg/dL, so she was referred to our hospital for further assessment and treatment. We immediately started hemodialysis. Later, high-titers of anti-glomerular basement membrane (GBM) antibody and MPO-ANCA were reported. We diagnosed vasculitis syndrome and added steroid pulse therapy and plasma exchange (PE). C-reactive protein immediately became negative but her platelet counts decreased, LDH elevated and schizocytes emerged. At that time her haptoglobin was undetected and ADAMT13 activity was low. Therefore, we clinically diagnosed TMA. Her platelet counts improved and LDH level decreased transiently while PE but after one course of PE her platelet counts decreased again. After several weeks, platelet counts gradually recovered and schizocytes disappeared. According to the clinical course, TMA may have been caused by steroid pulse therapy. In conclusion, nephrologists should be aware that steroid pulse therapies can induce secondary TMA.

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